Gastroenterology & Hepatology: Open Access Case Report Open Access A case of mistaken identity… Abstract Volume 5 Issue 8 - 2016 Paragangliomas are rare tumors of the autonomic nervous system, which may origin from Marina Morais,1,2 Marinho de Almeida,1,2 virtually any part of the body containing embryonic neural crest tissue. Catarina Eloy,2,3 Renato Bessa Melo,1,2 Luís A 60year-old old female, with a history of resistant hypertension and constitutional Graça,1 J Costa Maia1 symptoms, was hospitalized for acute renal failure. In the investigation, a CT scan revealed 1General Surgery Department, Portugal a 63x54mm hepatic nodule in the caudate lobe. Intraoperatively, the tumor was closely 2University of Porto Medical School, Portugal attached to segment 1, but not depending directly on the hepatic parenchyma or any other 3Instituto de Patologia e Imunologia Molecular da Universidade adjacent structure, and it was resected. Histology reported a paraganglioma. Postoperative do Porto (IPATIMUP), Portugal period was uneventful. Correspondence: J Costa Maia, Sao Joao Medical Center, A potentially functional PG was mistaken for an incidentaloma, due to its location, General Surgery Department, Portugal, interrelated illnesses and unspecific symptoms. PG may mimic primary liver tumors and Email therefore should be a differential diagnosis for tumors in this location. Received: August 29, 2016 | Published: December 30, 2016 Background and hydrochlorothiazide), was admitted to the Internal Medicine Department due to gastroenteritis and dehydration-associated acute Paragangliomas (PG) are rare tumors of the autonomic nervous renal failure (ARF). She reported weight loss (more than 15%), system. Their origin takes part in the neural crest cells, which produce anorexia, asthenia, polydipsia, polyuria and frequent episodes of 1 neuropeptides and catecholamines. In 10-50% of cases, they are muscle cramps, with 1year of evolution. She presented no risk factors hereditary, beginning at younger ages. In 80-90% of abdominal PG for chronic liver disease. She was the mother of 4, one of them and 5-10% of head and neck PG, catecholamine production can cause with Cushing’s syndrome. An abdominal ultrasound and angio-CT arterial hypertension, headache, palpitations, anxiety and weight was performed for the investigation of renal failure. These exams 1,2 loss. The diagnosis is usually made ​​by conventional imaging revealed a non-cirrhotic liver, with a heterogeneous hepatic nodule (CT, MRI, scintigraphy). A biopsy cannot distinguish benign from of 63x54mm in the caudate lobe. This nodule had a heterogeneous malignant tumors (<10%), which can be diagnosed in the presence contrast enhancement and included some areas of low density and 3 of distant metastasis. On the other hand, biopsy of these lesions enhancement (suggesting necrosis) (Figure 1). Liver function tests might be dangerous, with the possibility of causing a life threatening and tumor markers (CEA, CA19.9 and αFP) were assessed and 4 event. The most sensitive and specific pre-operatory diagnostic test unremarkable. Abnormal liver function could be present in the context 5 is the determination of plasma and urine metanephrines. Histological of cirrhosis or obstructive jaundice and a raise in tumor markers could examination showing polygonal cells with finely granular eosinophilic be interpreted in the context of primary or secondary neoplastic cytoplasm, oval nuclei and positivity for neuroendocrine markers disease of the liver. Upper and lower GI endoscopies were performed such as cromogranine A, neuron-specific enolase, synaptophysin and to exclude digestive primaries, which showed no endoscopic lesions. insulin-like growth factor II are specific for paragangliomas and are Surgical resection was proposed due to location-related difficulties in 6 not present in hepatocellular tumors. The treatment for non-metastatic obtaining a pre-operative biopsy. disease involves surgical resection.5 It is also recommended that these patients must be tested for succinate dehydrogenase mutations. Intraoperatively, after complete mobilization of the left hepatic lobe, the tumor was found closely attached to hepatic segment 1. It did In the non-cirrhotic liver, the most common benign diseases are not depend directly on the hepatic parenchyma or any other adjacent hemangioma, focal nodular hyperplasia (FNH), nodular regenerative structure, such as the inferior vena cava, adrenal gland or kidney. The hyperplasia and hepatocellular adenoma. The most frequent tumor was resected with no intraoperative incidents. malignancies are secondary tumors, cholangiocarcinoma and fibrolamellar carcinoma.7 The nodular lesion of 7x5x5,5cm and 74g exhibited a thin capsule and smooth surface. Histology revealed a neoplasm with an expansive Elevated αFP diagnoses hepatocellular carcinoma. growth pattern, constituted by very large epitheliod cells. Some of those Ultrassonography or CT identify cysts, metastases and hemangioma. cells were multinucleated, with granular and amphophilic cytoplasm, MRI is helpful in the diagnosis of focal fatty liver, FNH, hepatocellular and were constituted by pleomorphic nuclei with macronucleoli. adenoma and hemangioma. When the diagnosis is unclear, fine needle Some areas of peripheral necrosis were found. The mitotic index aspiration biopsy or follow-up imaging is considered. was very low. Adrenal parenchyma was not observed (Figure 2). Immunohistochemical study revealed cromoganine expression in the Clinical case absence of expression of AE1.AE3, which was consistent with the A 60year-old female with a history of Diabetes mellitus type 2, diagnosis of paraganglioma. under treatment with sitagliptin and metformin and severe arterial The postoperative period was uneventful and the patient was hypertension treated with 4drugs (nebivolol, lecarnidipine, olmesartan discharged on day 5, with only one antihypertensive drug (lisinopril Submit Manuscript | http://medcraveonline.com Gastroenterol Hepatol Open Access. 2016;5(8):119‒122. 119 ©2016 Morais et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and build upon your work non-commercially. Copyright: A case of mistaken identity… ©2016 Morais et al. 120 20mg/day). Two months later, a cervico-thoraco-abdominal-pelvic CT Discussion confirmed the absence of any suspicious cervical nodes of PG, adrenal glands without significant changes and no retroperitoneal, mesenteric, PG are more often located in the sympathetic chains from the neck pelvic or groin lymphadenopathies. Scintigraphy also showed no to the pelvis. However, unusual locations, such as the gallbladder, bile 8-10 evidence of any lesion bearing α-adrenergic receptors. In addition, ducts and bladder have been described. and in some reports, they 11,12 catecholamines in urine (vanillylmandelic acid, norepinephrine, may also mimic primary liver tumors. Tumors located in the liver 11,13‒17 epinephrine, dopamine, normetanephrine and metanephrine) were are extremely rare and only few cases are described. This is the normal. first case found in literature of a PG mimicking a primary liver tumor of segment 1. Autopsy studies have shown that, during life, 9.9% of people may develop a hepato-adrenal fusion, resulting from loss of fibrous tissue,18 which could explain the emergence of these lesions. They may be associated with the presence of ectopic cromaffinic tissue in the liver.16 This location may also be responsible, according to some authors, for the absence of symptoms. Since the venous drainage of the tumor to the portal system causes catecholamines to be metabolized by hepatocytes, their levels might not be elevated in the systemic circulation.12 In this case, the location, associated illnesses and unspecific symptoms have rendered the preoperative diagnosis of PG more difficult. Moreover, PG and hepatocellular carcinoma may have similar imaging features.4 In this case, a primary tumor outside the liver was first excluded. Then, due to the large size and location of the lesion in segment 1, which could pose difficulties for biopsy, surgical resection was proposed. This PG was an incidentaloma, which is the form of presentation in 25% of the cases.19 However, after a second-look on this case, the lesion was most probably an overlooked functional PG, due to its presentation with resistant hypertension, constitutional symptoms and also the higher prevalence of functional Figure 1 Nodule of 63x54mm mimicking a tumor of the caudate lobe. PG in the abdomen. Acknowledgments None. Conflicts of interest Authors declare that there are no conflicts of interest. Funding None. References 1. Erickson D, Kudva YC, Ebersold MJ, et al. Benign paragangliomas: clinical presentation and treatment outcomes in 236patients. J Clin Endocrinol Metab. 2001;86(11):5210‒5216. 2. Baysal BE, Myers EN. Etiopathogenesis and clinical presentation of carotid body tumors. Microsc Res Tech. 2002;59(3):256‒261. 3. Tischler AS. Pheochromocytoma and extra‒adrenal paraganglioma: updates. Arch Pathol Lab Med. 2008;132(8):1272‒1284. 4. Roman SA, Sosa JA. Functional paragangliomas presenting as primary liver tumors. South Med J. 2007;100(2):195‒196. 5. Plouin PF, Gimenez Roqueplo AP. Pheochromocytomas and secreting paragangliomas. Orphanet J Rare Dis. 2006;1:49. 6. Yeo H, Roman S. Pheochromocytoma and functional paraganglioma. Curr Opin Oncol. 2005;17(1):13‒18. Figure 2 Macroscopic aspect (A) and histological features (HeE,